A retrospective study of Creutzfeldt–Jakob disease in Belgium

Using data from Belgian neuropathological archives, completed with the results of a comprehensive study of available medical records, we found 100 patients who fulfilled diagnostic criteria for probable or definite Creutzfeldt–Jakob disease (CJD). Mean age at death was 63 years. The median disease duration was 9 months. Progressive mental deterioration was present in all cases, whereas signs of cerebellar dysfunction and myoclonus were found in approximately 80% of the patients. In 50% of the population, the EEG revealed characteristic abnormalities. Ninety-six patients suffered from the sporadic type of CJD, while 4 suffered from a hereditary form. In our series, we could find no evidence for the new variant, neither for an iatrogenic cause.     

Cerebral disseminated coagulation

Summary The distribution of cytarabine in the ventricular CSF was studied after lumbar intrathecal administration. In the ventricular fluid peak concentrations up to 1.5×10^–4 M (37 g/ml) were achieved 4–8 h after lumbar injection of cytarabine of 45 mg/m². The concentration of cytarabine decreased slowly with a half-time of 2.16 h thus providing therapeutic concentrations in the cerebral ventricles for more than 24 h.

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Zusammenfassung Die Verteilung von Cytosinarabinosid nach lumbaler intrathekaler Injektion im ventrikulären Liquor cerebrospinalis wurde untersucht.Nach lumbaler intrathekaler Injektion von 45 mg Cytosinarabinosid (Alexan®) pro m² Körperoberfläche wurde im ventrikulären Liquor eine Höchstkonzentration von 1,5×10^–4 M (37 g/ml) 4–8 h nach Applikation erreicht. Die Konzentration von Cytosinarabinosid im ventrikulären Liquor fiel langsam mit einer Halbwertszeit von 2,16 h ab. Bei lumbaler Applikation wurden bei dieser Untersuchung therapeutische Konzentrationen von Cytosinarabinosid in den cerebralen Ventrikeln für mehr als 24 h erreicht.

     

Intraosseous neurilemmoma of the radius: a case report

Neurilemmomas are benign tumors that arise from Schwann cells. They are rarely found on bone. We describe a neurilemmoma in a 45-year-old patient that affected the distal metaphysis of the radius. Only 1 previous case has been described in the literature. We discuss the clinical presentation, the radiographic aspect, particularly the magnetic resonance imaging characteristics, and the microscopic findings.     

Review: Clinical Variability and Genetic Heterogeneity in Multiple Epiphyseal Dysplasia

This review reports on multiple epiphyseal dysplasia (MED), first described clinically in the early part of the 20th century. Over 50 years later, we are now beginning to unravel the mystery behind the genetic mutations involved in triggering the changes in cartilage observed in this condition. In the past decade considerable progress has been made in identifying the underlying genetic defect in some forms of MED. Understanding the precise effect that these molecular changes have on the integrity of the cartilage extracellular matrix will lead the way in identifying the complex disease pathophysiology that defines MED. In addition, a greater understanding of the role and interactions of specific cartilage molecules may reveal the basis of more widespread cartilage disorders such as osteoarthritis.     

Absorptive hypercalciuria type III or caused by renal loss of phosphates

Contribution of a retrospective series of 16 patients with type II absorptive hypercalciuria over a total of 1.041 patients undergoing metabolic study due to relapsing renal lithiasis. Clinical history of lithiasis, biochemistry prior to treatment and instituted therapy were examined in all cases. Stones composition, radiologic appearance of lithiasis and evolution of biochemical parameters after medical treatment with a mixture of phosphates were also studied.     

Expression of Adhesion Molecules and Cytokines on Saphenous Veins in Chronic Venous Insufficiency

The objective of this study was to assess the relationship of signaling molecules to monocyte/ macrophages as a precursor to venous valve and venous wall dysfunction in patients with varicose veins. One of the hallmarks of venous dysfunction is destruction of venous valves with subsequent reflux and elevation of distal venous pressure. We recently observed that monocytes/macrophages migrate into the venous walls and valves of patients with venous insufficiency. There, they may play a role in the pathogenesis of primary venous insufficiency. If so, an important element in their performance would be the interaction between the monocytes and the endothelium as a precursor of damage to venous valves and the venous wall. To explore this interaction, immunohistochemistry was carried out to detect adhesion molecules and cytokines in surgical specimens removed during surgical therapy. Twenty-four surgical specimens consisting of proximal saphenous vein and subterminal valve were obtained using minimally traumatic technique in 6 males and 18 females who ranged in age from 31 to 79 years. Reflux was confirmed preoperatively by duplex technique, and severity was classified by the CEAP classification of the American Venous Forum. Ten patient limbs were class 2, eight were class 3, four were class 4, and two were class 6. The venous specimens were labeled using monoclonal antibody against ICAM-1, E-selectin, IL-1alpha, and TNF-alpha. CD68 was used for detection of monocytes/macrophages. Our results indicate that not only luminal venous endothelium but also endothelium in the vasa vasora of refluxing saphenous veins is activated, as indicated by the up-regulation of ICAM-1. However, IL-1alpha and TNF-alpha were increased in only selected specimens and are mainly detected in the vein wall. The factors that serve as trigger mechanisms to activate cells in the pathogenesis of primary venous dysfunction remain to be explored.     

Molecular and functional characterization of porcine LFA-1 using monoclonal antibodies to CD11a and CD18

Abstract: We describe in this report the production and characterization of monoclonal antibodies (mAb) to the swine homologues of CD11a and CD18 antigens, and their use for phenotypic and functional analysis of porcine leukocytes. Monoclonal antibodies BL1H8 and BL2F1 precipitated two bands of approximately 170 and 95 kDa, whereas mAb BA3H2 brought down three bands of 170, 155 and 95 kDa, from alveolar macrophage lysates. Clearance of macrophage lysates with mAbs BL1H8 and BL2F1 resulted in complete removal of the 170-kDa band. The cell distribution of the molecules recognized by these mAbs was similar to that of human LFA-1. It was found on all leukocytes, although its expression varied among the different leukocyte subpopulations, with monocytes, granulocytes and a subset of CD8⁺ cells expressing the highest levels. Cross-blocking studies showed that these antibodies recognize different epitopes on porcine LFA-1. Both anti-LFA-1 mAbs strongly inhibited the mitogenic response of PBMC to ConA, whereas the anti-CD18 mAb had no effect. These anti-LFA-1 mAbs also inhibited the mixed lymphocyte reaction (MLR) and the NK cell-mediated lysis of K-562 cells.     

Subcellular compartmentalization of a potassium channel (Kv1.4): preferential distribution in dendrites and dendritic spines of neurons in the dorsal cochlear nucleus

Voltage-dependent ion channels have specific patterns of distribution along the neuronal plasma membrane of dendrites, cell bodies and axons, which need to be unravelled in order to understand their contribution to neuronal excitability and firing patterns. We have investigated the subcellular compartmentalization of Kv1.4, a transient, fast-inactivating potassium channel, in fusiform cells and related interneurons of the rat dorsal cochlear nucleus. A polyclonal antibody which binds to a region near the N-terminus domain of a Kv1.4 channel was raised in rabbits. Using a high-resolution combination of immunocytochemical methods, Kv1.4 was localized mainly in the apical dendritic trunks and cell bodies of fusiform cells, as well as in dendrites and cell bodies of interneurons of the dorsal cochlear nucleus, likely cartwheel cells. Quantitative immunogold immunocytochemistry revealed a pronounced distal to proximal gradient in the dendrosomatic distribution of Kv1. 4. In plasma membrane localizations, Kv1.4 was preferentially present in dendritic spines, either in the spine neck or in perisynaptic locations, always away from the postsynaptic density. These findings indicate that Kv1.4 is largely distributed in dendritic compartments of fusiform and cartwheel cells of the dorsal cochlear nucleus. Its preferential localization in dendritic spines, where granule cell axons make powerful excitatory synapses, suggests a role for this voltage-dependent ion channel in the regulation of dendritic excitability and excitatory inputs.